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H & N Symptoms in Neurologic Disease: What to Look For

Katherine C. Yung, MDAssistant Professor, Division of Laryngology

Dept. of Otolaryngology-Head and Neck Surgery

Basic Neurological Motor Pathway

� Pyramidal Motor System� Upper Motor Neurons (UMN)

• Descending Pathways – Pyramidal Tracts• Corticospinal tract• Corticobulbar tract

• Activate the lower motor neuron

� Lower motor neuron (LMN)• Peripheral motor nerves

• Spinal• Cranial (Bulbar)

� Neuromuscular junction� Neurotransmitter (acetylcholine) released from nerve terminal

flows across junction and stimulates muscular contraction

� Muscle

Neuroanatomic pathways Symptoms suggesting Neuropathology

� Speech � Dysarthria, hypernasality, abnormal

resonance

� Voice� Asthenia, breathiness, instability, strain

� Swallowing� Oral incompetence, aspiration, nasal

regurgitation, inability to initiate swallow

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Clinical Assessment� Basic head and neck

exam, including cranial nerves� Special attention to:� Facial and lateral jaw

movements� Tongue fasiculations� Tongue strength� Coordination of tongue

movement� Laryngeal elevation with

swallow� Velar function

Clinical Assessment

� Perceptual speech and voice evaluation� Laryngeal Exam� Vocal fold motion� Pharyngeal wall

motion

� Consider: FEES or MBS

Extrapyramidal Neurologic System

� System of nerve tracts and pathways connecting the cerebral cortex, basal ganglia, thalamus, cerebellum, reticular formation, and spinal neurons in complex circuits not included in the pyramidal system

� Responsible for coordinated reflex interactions

� Affects motor function by either facilitation or suppression

Extrapyramidal Neurologic System� Voice� Hypotonic – flaccid� Hypertonic – constricted

� Speech� Spastic� Ataxic

� Breathing� Vocal fold dysfunction (paradoxical motion)

� Swallowing� Impaired if associated with significant

muscular weakness

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Associated Symptoms

FAILURE TO SUPPRESS

� Tremors� Chorea� Athetosis� Dystonia� Myoclonus

FAILURE TO FACILITATE

� Bradykinesia� Diminished postural

responses

Spasmodic Dysphonia� Voice� Increased effort� Unreliable in different situations (Stress)�Whisper is normal� Maybe able to sing� Abductor and Adductor varieties

• Patients usually aware of words and situations which make voice worse

� Swallowing - Uninvolved

Spasmodic Dysphonia Spasmodic Dysphonia

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Vocal Tremor� Voice� Tremor� Strain/roughness� Often deny effort associated with SD� Not sound specific

� Swallowing� Unaffected

Tremor

Parkinson’s Disease� Voice�Weak with early fatigue� Breathy - soft� Pitch elevated

� Speech� “mumble”

� Swallowing – potential problems late in disease

Parkinson’s Disease

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UMN Pathway Disruption

� Spasticity� spastic dysarthria� spastic dysphonia

� Swallowing and other vegetative functions-relatively well preserved until disruption is severe� Swallowing - Inability of UES to relax� Breathing - Inability of vocal folds to relax to produce

voice or allow inspiration

UMN Pathway Disruption

LMN Pathway Disruption� Flaccidity� flaccid dysarthria� flaccid dysphonia

� Swallowing and other vegetative functions are affected early� Dysphagia to liquids� Breathing – impaired due to lack of

abduction

LMN Pathway Disruption

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Associated Signs & Symptoms

� Upper motor: spasticity, hypertonia, hyperreflexia, clonus, Babinski sign

� Lower motor: flaccidity, hypotonia, hyporeflexia, atrophy, fasciculations (usually for motor neuron disease only)

Site of Lesion� Extrapyramidal disorders� Parkinson’s disease� Cerebellar stroke� Spasmodic dysphonia� Tremor

� Upper motor neuron disorders� Stroke � Pseudobulbar palsy� Primary lateral sclerosis (PLS)

Site of Lesion

� Lower motor neuron� Brainstem stroke (e.g. lateral medullary syndrome)� Myasthenia gravis� Guillain-Barre’

� Polio (post-polio)

� Mixed� TBI� Motor Neuron Disease

• ALS• Progressive Bulbar Palsy

Motor Neuron Disease

Type UMNdegeneration

LMNdegeneration

ALS yes yes

PLS yes no

PMA no yes

Progressive bulbar palsy

yes - bulbar region

yes - bulbar region

Pseudobulbar palsy

yes - bulbar region

no

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Motor Neuron Disease in the Otolaryngology Clinic

� 1759 patients presented with voice, speech and swallowing complaints� 15/1759 diagnosed with ALS� Referring diagnoses included

• Unknown neurological disease• GERD• Stroke• Bowing• SD• Polyp

� 220 patients diagnosed with MND in Neurology clinic� 44/220 presented with bulbar signs (dysarthria, dysphagia, dysphonia) � 19/44 initially presented to otolaryngologist� 8/19 neuromuscular disease was missed initially by ENT

Chen, A, Garrett CG. Otolaryngol Head Neck Surg. 2005 Mar; 132 (3):500-4.